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Wednesday, June 24, 2015

While I did received some bad news this week, so many wonderful things have been happening in my world! ...Wow lets just take a moment to appreciate that. Those are not words I get to type very often! While my health may still be struggling, as it always will be, I finally feel I am on a solid treatment plan that gives me enough energy to at least enjoy a few small victories! My weekly iron infusions have made a huge impact on my overall energy levels and improved my quality of life immensely! Plus I no longer have the coloring of a zombie! lol It feel so good to have iron pumping through me veins again!

I had another dosing of the chemotherapy Rituxan (Rituximab) which suppresses my B-Cells and essentially obliterates my immune system. This drug is helping to suppress my disease and I FINALLY feel like it is working! With this new more frequent dosing I can almost see and feel the differences in my skin, breathing ability, and organ function! I have been able to start drinking things again which is amazingly satisfying and I believe it is a side product of the drug. I am hoping to work up to small amounts of puree items but that will only come in time if my body is willing. Disease is a tricky and conniving little demon... it may decide that liquid is off limits again at any moment, but lets hope not! I feel my ability to breath has improved slightly too; my doc said my lungs do sound a little less stressed! Woohoo!

I kinda feel like I live at the infusion center currently; I have made some pretty good friends with the nurses after seeing them every five days! One of the nurses that watched my Port access last time got to use me as her first ever Port access patient! She did so well and got a perfect access!

I had an appointment with my Rheumatologist who was very satisfied with my progress! Between the new drugs, more rigorous treatment plan, and use of oxygen I seem to be really holding my own. One thing I want to clarify for my readers is that my lungs currently are fine-ish. The reason I am on supplemental oxygen is due to the poor function of my respiratory muscles. The respiratory muscles that are responsible for expanding the lungs do not work adequately leaving my under oxygenated with activity such as walking or talking for long periods of time. My actually lungs do not currently have mass amounts of fibrosis, I actually have basically none; it's my muscles that are causing the dip in my oxygen levels requiring the use of supplemental oxygen. You will not see me on oxygen 24/7 but will see me on it when I am walking out and about, at the mall, or out for a long day with family. If I am engaged in an activity where my body will be at rest for a long period of time such as watching a movie, driving, or sitting on the couch, I will more than likely have fine oxygen levels and not require its use, however sometimes I might. My body knows when I need it and when I don't.

I also had an appoiment with my Gastroenterologist this week. Since my body has decided that SIBO (small bacterial intestinal overgrowth) is my new normal I am officially set to be on antibiotics the rest of my life. While this is not optimal, it is the only way to combat SIBO, and is much better than the alternative -- diarrheaing out all your nutrition and iron, which requires hospitalizations every other week. I will be on a alternating daily schedule of heavy antibiotics: one day on, one day off to tackle this overgrowth and hopefully settle my bowls. This issue is what is causing my serve anemia as my bowls can no longer absorb iron. We are hoping that by counteracting the SIBO with antibiotics on this heavy regiment that my iron will level out so I can discontinue my infusions every five days. As of now we will be stopping the iron infusions until we know if the antibiotics are working; if I still in 6 weeks can't keep up my iron levels we will be starting frequent infusions again. Since all of this seems to be spiraling out of control lately my doctor ordered a "Smart Pill" test that will track my digestive system transit time. I basically just get to swallow a giant pill (which may actually have to be placed through an endoscopy since my esophagus doesn't work) which will decipher where my intestines have slowed/stopped so we can find the right medication to stimulate that particular area of the tract.

Lastly I had an appointment with a Cardiologist to follow up with some palpitations I've been having, and a small murmur that has recently shown up in clinic. This new cardio went over my history, and old Holter Monitors results that track the rythem and beats of the heart; what he found was quite surprising. All of my monitors over the past year have showed multiple episodes of SuperVentricular Tachycardia (SVT.) This can be a serious arrhythmia due to improper electrical activity in the heart causing a plethora of problems including fainting, dizziness, lowered blood pressure, chest pain, and a decrease in cardiac output. The doctor was pretty peeved that these test results were not read properly, and that I was not informed of this issue; SVT clearly correlates with all my symptoms. Luckily, this is an easy problem to fix with a medical procedure called an Ablation. An ablation is preformed by placing a long thin catheter through a blood vessel in your groin into your heart where a piece of the tissue is modified using Radio Frequency, essentially re-wiring the heart to stop beating incorrectly. While this sounds very big and scary, it is supposedly a simple procedure that has very high success rates! He told me that I will feel much better once my heart is re-wired to beat properly! We haven't scheduled this procedure yet, as we want to do a few more tests before we move to this somewhat drastic measure. For now I will re-preform a Holter Monitor for 24 hours, and follow up with him to address the results in two weeks.
As a interesting side note, he expressed to me that my small murmur is due to a "heart string." My Left Ventricle has a small chord running across it that causes innocent murmurs; apparently it's completely benign and is an abnormality formed at birth.

A very BIG thank you to all the supporters who came out for the Scleroderma 5k last weekend! I cannot begin to tell you how much it meant to me to have so many people walking by my side! Our team had the largest number of members; 35 strong! I am still so blown away by the love I have received from our small community! I walked almost the whole 5k, but alas, my poor body couldn't make it all the way. Thankful my husband Noel made it up for me! ;) Running a 10k in my honor! I love that man so much!

In some other rather exciting news I have been asked to be a speaker at this years Scleroderma National Foundations Patient Education Conference! I will be one of three patients on a panel talking to others about "Living Well with Scleroderma"! I am so excited for this opportunity and beyond flattered that the Foundation thought to ask me of all people! Now I am even MORE excited to attend next month! I would love to meet all of my readers at the conference if you will be attending! Look for my panel in the schedule on Sunday in the Belmont Workshop Room from 11-12pm!

Saturday, June 13, 2015

Life is a fragile thing. In an instant it can be destroyed by a circumstance beyond your control; the existence you knew shattered like glass beneath your feet. No matter how hard you attempt to piece back the fragments of that previous life there is no restoring it to exactly the same as before.
I remember my life fracturing moment vividly; it consisted of one hour that changed the entire course of my being. October 21st 2011 I was diagnosed with an incurable, and fatal illness called Systemic Scleroderma. I remember walking into that Rheumatologist office equipped for the worst, but still being unprepared for the news I received. In that hour I was told I would be fortunate to live another ten years -- and that was only if my circumstances were favorable.

At the age of 20 I should have had engagements such as college, relationships, and my career swirling through my mind, instead I had my own mortality. I left that office with pamphlets to read, treatments to start, and a new way of life to begin. I was warned of the path my disease would take, and of the horror I would soon endure. My health forced me to watched idly by as my fellow high school graduates left for college, received their degrees, started budding careers, and even began their families. As they progressed through the natural events of life I was alternatively attached to events such as chemotherapy, long hospital admissions, support group, and never ending chronic pain. Slowly over a course of three years my body began to deteriorate, vital systems starting to shut down, and organs beginning to fail. By the age of 23 I was being kept alive by a surgically placed feeding tube, supplemental oxygen, dangerous chemicals, and a team of specialized physicians.

As a young adult you have so many goals for your life, and you honestly believe you have 100 years to achieve them. Suddenly I was given a sliver of that time, and a body that could not handle even the simplest of tasks. I focused on nothing but reclaiming my shattered pieces, of sweeping up and attempting to place them exactly the same as they had been before. I believed I could attain that pre-diagnosis life if I just situated all the shattered pieces back together -- but I quickly found out that that is not the way life works. Many of my pieces no longer existed, crushed beyond what I could realistically repair. Just like shattered glass I found I could not perfectly align the fragments to resemble the flawless and unbroken product. I however during my journey discover that those splintered pieces could be fashioned into something quite new and extraordinary. While this current finished product may have cracks, protruding edges, and be held together by messy glue, it has certainly never been more dazzling.

True joy is letting go of what you believe your life is intended to be, and instead revering all that it is. Once I accepted this new existence, alone with every alteration and imperfection, I began to enjoy my life again. I began to enjoy who I was again. I no longer focused solely on recovering what I had lost, but on creating something new. I focused on the small victories and minute satisfactions found in my day to day routine. I took the time to enjoy every breath, every word, and every moment. Although my existence doesn't seem like anything to be cheerful about to the typical observer, I have found so many incredible moments to revel in. My life may not be glamorous, or as long as I intended it to be, but it is one full of simple pleasures and an understanding of how broken things can still be beautiful.

Friday, June 12, 2015

This last weekend I had the wonderful opportunity to spread some Scleroderma Awareness in Seattle at The Great Wheel which was lit up in Teal for Scleroderma Awareness Month! Our Washington State chapter is such a great group of individuals dedicated to sharing knowledge about this disease.
Advocacy work such as this is truly my passion, and even though not one person was interested in learning more about the disease during their sunny excursion on the pier, I still had a blast! I even met Spider Man ;)

As many of you know I was hospitalized last week due to a quick onset of anemia. I have never before encountered this ailment and my doctors are quite puzzled as to why I have a sudden onset. My Iron, Hemoglobin, and Ferritin, seem to be plummeting out of the blue. We will probably have to do some further follow up such as an internal scope to check for possible internal bleeding as I must be losing all this blood from somewhere in my body. This issue can be quite common in Scleroderma patients so my doctors are not surprised, especially with all the bowl issues I have encountered lately. Unfortunately the SIBO situation has not improved even on the heavy antibiotics so my GI may have to come up with a different game plan in the near future; I see her again at the end of this month.
This week I started my iron infusions, as of now my doctor has ordered them to be received every five days! I am hoping that as I stabilize we can move to a more tolerable frequency such as once a month. I am receiving the infusions at a new center down the freeway from my house so I dont have to trek all the way to the university every five days. This new center is amazing and I am totally in love with all the nurses! Everyone was so friendly and excited to have a patient with a Power Port to practice on! I believe I was their first patient with a Port to arrive at this newly opened center. Luckily, they had a seasoned Port nurse, but all the rest had never accessed one before but were eager to learn and watch! It was rather fun having them all huddled in my small room to get a better look, I felt like I was giving a presentation to a class ;) And you all know how much the teacher in me loves that! The infusion went smoothly. Iron is odd because it looks like tar and leaves an odd caramel taste in your mouth. Luckily the infusions are very short and I am only at the center about an hour, so I was quickly on my way home! I let one of the excited young nurses de-access my port, she was so grateful to have a laid back patient to practice on -- while many people would call this "being a guinea pig" I truly don't think of it that way. I enjoy giving learning opportunities to the awesome medical professionals that take such good care of me. I loved how happy the nurse looked when she pulled out that large needle successfully!

I also wanted to take this opportunity to personally thank each and everyone of you who donated to my GoFundMe account. I have officially raised enough money to purchase a Portable Oxygen Concentrator! I am now free of heavy tanks that hinder my ability to partake in my favorite activities such as swing dancing, hiking, and traveling. I now have the ability to travel on planes, trains, and ferries; no longer locked in to my small hometown. I am so excited to start this new journey of breathing easy! Thank you all from the bottom of my heart! You have literally given me the gift of adventure!

Next week will be a busy one! I have another Iron Infusion, a Rituxan Infusion, and an appointment with my Rheumatologist; however, Saturday is the day I have been waiting for! The Seattle Scleroderma 5k will finally be here! Please contact me by email for more information regarding the walk or visit Scleroderma.org/washington and follow the 'special events' link to the Seattle "Into The Feature" walk! I look forward to seeing all of you there supporting myself, and this amazing cause!

Wednesday, June 10, 2015

When I started this site my goal was to raise awareness for my unheard of illness (Systemic Scleroderma), but mostly to give a glimpse to the general public of what day to day life looks like for a patient battling serious illness. I wanted to bring to light the misconceptions around living with disease, and to express my honest thoughts about this life style. I was not looking for popularity, sympathy, or any compensation when beginning to journal my every day reality; I was simply looking for an emotional outlet.
As my posts have now become viewed by thousands I have found that the average Internet goers are exceptionally vulgar and lacking any empathy at all. They apparently have the inability to sit back and accept another's opinion, or pretend for a moment they are in the authors shoes. I honestly have to sit back and laugh at comments I have read on sites such as Yahoo, The Huffington Post, and many others! People feel entitled behind a screen, they will never get caught, and never have to face a consequence for their words. They feel victorious when they slash the keyboard attempting to bring down the individual that has barred their soul for the world. We seem like weak and vulnerable targets to these over opinionated bullies who have nothing better to do.
Please know I take these 'comments' with a grain of salt, and many of them I don't even read. Everyone is entitled to their opinions, just like I am mine. I have every right to share my thoughts on the life I must endure -- just like they have every right to comment on it. Now before you accept that as an invitation to demolish my existence take a moment to picture yourself in my shoes.

Imagine you are a 23 year old young woman with no life ahead of you. You are attached to a heavy oxygen tank because you cannot breath properly on your own; you feel crushed and suffocated with every breath. Imagine your nauseous at all times, you want to eat food but either it gets lodged in your esophagus, or you vomit wildly like you have food poisoning with even the smallest of bites. Imagine you have a large tube protruding out your stomach that you receive all your nutrition from -- that you can no longer eat. That cheese burger you just saw on the Red Robin commercial will never be yours. You want your favorite salad from Olive Garden. No.
Imagine every six weeks getting a one inch long needle shoved into your chest for chemotherapy. Imagine the 28 prescriptions you take three times a day. Imagine the crushing, solidifying feeling of constant joint pain with every move.
Imagine you are me.
Just five years ago you had your whole life ahead of you, anything was at your finger tips. You had a college scholarship and was ready to follow your dreams, marry your high school sweetheart, have children, buy a house, and live a beautiful life till you were old and gray.

That fantasy is gone, and a harsh reality has taken its place. It was swept away in an instant and your life is over before it ever began. You're told you are going to die, you can never have children, and will lead a miserable, painful existence for the remainder of your days full of doctors appointments, surgeries, infusions, fistfuls of medications, and harsh chemotherapy.
Now that you have a brief understanding of my world, think about your life behind that keyboard. How old are you? How many children do you have? Did you get to attend college? Can you eat? Can you breath? Are you able to work? Can you walk without pain?
These are things I will either never get to participate in, or likely will ever do again. The purpose of this post is not to complain about my life, (as I actually quite adore it) but to give you a better chance at comprehending who you are slamming on the other side of that story.

Now, I invite you to please tell my cannula wearing, feeding tube wielding, chemo-riddin self what you think of me if you still feel 'big and tough' behind that computer screen. I am a strong woman and if a terminal illness doesn't get me down, your words surely will not!
If you have nothing better to do than poke inappropriate, narcissistic comments at a sick young woman who can't defend herself you should probably take this opportunity to do some serious soul searching. That being said...

With that negativity out of the way I also wanted to share for a brief moment the incredible side of the Internet I have found through blogging! I have received dozens of heartfelt emails, comments, and messages expressing support for my condition, and encouraging me to continue my work raising awareness. Each of these emails make every single negative comment melt into the background. Thank you from the bottom of my heart to all of those readers who take the time to personally find out a little more about who I am, and realize that there is an actual living person behind this screen!

To learn more about me please visit the "Welcome" and "My Story" tabs located on the top navigational bar. To learn more about Scleroderma please visit the "Sclero-What?" tab.

Scleroderma (Scl-eh-roh-derm-uh) is greek for hard-skin, but this hell of a disease is so much more than that. Take a moment to imagine having thick concrete slowly poured into your skin, your blood vessels, your organs; horrifically crushing and solidifying you until movement becomes impossible.

This devastating vandalism of the body is a result of an over production of collagen, a connective tissue protein that holds us all together. When overproduced the collagen becomes thick, fibrous scar tissue throughout the patient, crushing their blood vessels, paralyzing organs, and hardening the skin -Essentially turning you into a living statue. Like many other rheumatic disorders, Scleroderma is an autoimmune disease because the condition triggers the body to make antibodies against itself. Chronic, complex and debilitating, this disease can affects the internal organs with life-threatening consequences. In its systemic forms scleroderma can damage the lungs, heart, kidneys and gastrointestinal tract with grave results. Vascular damage due to a loss of circulation and oxygenation can result in tissue death, severe ulcerations, and even a loss of digits. In many cases, the joints and muscles are affected, resulting in widespread chronic pain and limited mobility. Pain, ranging in severity from uncomfortable to unbearable, is a common characteristic of the disease.

The skin tightening alone can leave patients physically unable to move their extremities, or in severe cases even entire limbs. These patients bodies are permanently contracted and disfigured leaving them unable to preform simple tasks such as opening a bottle, putting on their shoes, pulling up their own pants, or brushing their own hair. This disease changes everything you knew about yourself, and strips you of the dignity to do the most basics of life's tasks.

Many patients actually resemble each other because of the resulting facial features; large rounded eyes, red blotchyrashes, receded lips, and skinnied noses.

There are multiple types of Scleroderma:

Localized Scleroderma: (Morphia & Linear) Which effects the skin, muscle, and joints, but does not normally effect the internal organs. In very rare cases it has effected the GI tract and brain in patients with 'En Coup De Sabre'. This form is still extremely debilitating, especially in children, as it can hinder their normal growth and development.

Sine Sclerosis: Which effects only the internal organs and not the skin.

Systemic Scleroderma: (Systemic Sclerosis & CREST): Which effects the skin, joints, muscles, vascular system, and internal organs. There are three subsets of systemic scleroderma which effect the organs at different progression rates: limited, overlap, and diffused

Today, there is no way to prevent any type of scleroderma and there is no cure. Treatments are available for some, but not all of the most serious complications of the disease. Current treatments include medications that suppress or modulate the immune system, chemotherapy drugs, vasodilators, Proton Pump Inhibitors, and ACE-inhibitors. Other treatments options include the placement of feeding tubes, the use of oxygen, surgical procedures, or Stem Cell Transplant/therapies. Presently, most treatments act to slow the progression of the disease and limit damage rather than truly arresting the disease or fixing existing fibrosis. In addition, some of the drugs currently in use can have serious side effects. Patients are normally seen by teams of physicians who assist in the management of different organs, and body parts effected. Treatments at best help ease discomfort and prolong life expectancies, though prognosis is usually poor in the systemic forms of the disease ranging anywhere from a few months, to 20 years.

How Does Scleroderma Personally Effect Me:

Scleroderma is what many call a 'designer disease', it effects each of its victims entirely different. While many of our symptoms and characteristics may be similar, we all truly have unique journeys.

Scleroderma has effected my skin minimally, leaving me with a thick hide instead of truly hard accustomed to most Scleroderma patients. My fingers are stiff and unable to straighten no matter how hard I push them together; my face, lower legs, and lower arms have also thickened to the point where I feel somewhat like stiff silly putty as opposed to hard plastic like many Scleroderma sufferers. For me this disease has greatly affected my internal organs and vascular system. The soft muscle tissue organs such as the esophagus, stomach, intestines, colon, and respiratory muscles have hardened, leaving them unable to function adequately. Image taking the esophagus, a soft wiggling organ that uses peristalsis to gently move food to your stomach turning to rock; no longer able to push food along in waves as it is so stiff to wiggle or contract. Imagine the stomach no longer able to empty, leaving you feeling like you have food poisoning every time you try to eat, violently vomiting as the body rejects the food it cannot move through its system. My digestive system has literally turned to stone, no longer able to do its job; no longer like a sponge that can absorb nutrients, but a solid mass that nothing can penetrate. Imagine the diaphragm no longer able to expand properly leaving your lungs unable to fill to their full potential; shortness of breath following your every foot step.

My vascular system has been crushed by this extra collagen leaving me horribly under oxygenated in my extremities due to a lack of circulation. My finger tips literally die, turning into ischemic ulceration that must be surgically amputated. Even when I do not feel cold my hands, feet, and even knees, wrists, nose, lips, and tongue will turns a violent shade of purple. This vascular issue is not only found in the visible vessels, but the organs as well leaving breathing difficult when I become cold.

Scleroderma has taken my once active life and left me with a husk of a being, unable to preform simple tasks such as eat, breath, or button my own blouse. I found myself stepping down from a promising career as an educator due to my physical limitations and being placed on State Disability at the young age of 23. I am fed by a feeing tube surgical placed in my stomach, breath supplemental oxygen through cannulas placed in my nose, and am pumped full of dangers chemicals through a Central Line in my chest that keeps me alive another few years. While this disease has literally made my existance a living hell, it has provided me with an incredible view of life one only receives through immense suffering and pain. For more information regarding my diagnosis please visit the 'My Story' page located across the top navigational bar.

My Current set of daily pills, liquid supplementations, and devices.

My tightening skin leaves limited mobility in my fingers.

How Does Scleroderma Effect Other Warriors:

My incredible friend Jessica M, and founder of Scleroderma Strong, has bean effected in a much different manner than I; Jessica battles severe skin tightining. Since I do not have much expertise in this area of the disease I asked her to share what it is like to live with this ailment, and to share a few photos to enlighten readers of Scleroderma's physical effects.

"When I was first diagnosed with Scleroderma, 4 years ago, I had no idea it would advance to the stage I am at now. Originally I was diagnosed with Limited Scleroderma, which means the tightening is limited to certain areas of the body. But as time went on I became worse and advanced to Diffuse Scleroderma, which is a lot of skin involvement, included with some organ involvement. My skin is so tight, it cannot be pinched! Imagine that. The worst area it has affected is my hands, which isn't surprising because my hands were the very first place I experienced tightening, 8 years ago. My fingers are stuck in a claw-like position, making everything impossible to grip. I can barely hold a cup, fork, sandwich, book, turn the steering wheel, etc. Not to mention the ulcers on my knuckles! My wrists barely move forward or backward. My shoulders are so tight, I cannot lift my arms above my head. The skin around my neck is so tight, I can hear it cracking when I turn my head from side to side. It is essentially like I am frozen in place, with little room for dexterity. So day-to-day life can be very difficult! But my family helps me with everything. Without my support system of good friends and family, I wouldn't make it through the day!"

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Woman and men like me, like Jessica, and like so many others battle this disease day in and day out; as she once so bravely put on her website, "your worst nightmare our my reality." Each and everyone of us work to bring awareness to this unknown ailment that strikes our fragile bodies; we fight for our lives, but also for our sisters and brothers battling by our sides -- We are too young to be lost to these disease.

The three major Scleroderma organizations in North America, The Scleroderma Foundation, The Scleroderma Research Foundation, and The Scleroderma Society of Canada, have created the "Hard Word. Harder Disease." campaign for the purpose of spreading awareness for this horrific disease. If you do one thing this month, please let it be to assist them in their campaign. Wear a pin, post on your Facebook, share this blog post, and take the Pledge! Visit HardWord.org and take the pledge to support Scleroderma Awareness this month! Each and every signature counts as one more person 'Scleroderma Aware.' With awareness comes funding, with funding comes research, and with research comes a cure!

Tuesday, June 2, 2015

While many pictures of me will show little evidence of illness, let me assure you, being sick is not a glamorous existence. Without make-up, stylish clothing, the concealment of my medical devices, and the repression of specific information regarding certain unpleasant symptoms, my life is actually quite vulgar. Hospital stays like my most recent are the true epitome of my life. This excrussiatingly real existence I attempt to keep out of the light of others because it is very unpleasant and not exactly appropriate conversation to strike up with others. No one truly wants to hear about your leaking body parts, horrendous rashes, or infected stoma drainage that ruins all of your clothes.

Sunday night after another beratement of heart palpitations, explosive diarrhea, weakness, dangerously low O2 saturation, excessive bruising and extreme paleness, I found myself in the Universities ER for the second time in ten days. My ragging case of SIBO had once again depleted my electrolytes, this time causing moderate anemia. My bowel moments had become so frequent that making it to the restroom was not an option. A lovely symptom of Scleroderma is decreased pressure in the different sphincters of the body, suprisingly you have more than just the one; there are actually over 60 in the human body. So when you have diarrhea and Scleroderma, you better have a change of pants. Now are you getting the picture why my life is less than glamorous?

After being admitted for electrolyte and iron infusions my levels had risen to stable and I was aloud to return home on antibiotics after one night in the hospital. Due to this consistent issue with electrolytes, anemia, and reoccurring SIBO I will have to start Iron infusions weekly. I will be working out the details with my GI when i see her in a few short weeks; hopefully I can stay out of the ER until then.

So when you see pictures of me smiling, looking well put together, or wearing a stylish outfit remember, that is not an authentic representation of my life... and those probably weren't my first pair of pants for the day.